CTICasesPulmonary Light-Chain (AL) Amyloidosis with Progressive Bullous Emphysema
Imaging findings
Sequential CTs over one year in a patient with known AL amyloidosis show progressive basal-predominant hyperinflation with large bullae expanding to occupy entire lobes and exerting mass effect on adjacent lung. The emphysematous spaces become exuberant and replace nearly the entire lower lobe volumes over the course of approximately 12 months.
Key takeaways
Pulmonary AL amyloidosis can cause a form of cystic and emphysema-like lung destruction through amyloid deposition that digests elastic tissue, producing pan-lobular emphysema with bullae formation. Progressive hyperinflation in the setting of amyloid deposition is a hallmark, and the mechanism involves preferential air filling of highly compliant, elastically digested lung spaces (not true positive-pressure overinflation). This is analogous to bullous emphysema mechanics described by Boushy et al.
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